Orphanet: Hereditary diffuse gastric cancer
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Hereditary diffuse gastric cancer

Disease definition

Hereditary diffuse gastric cancer is a rare epithelial tumor of the stomach, characterized by the development of diffuse (signet ring cell) gastric cancer at a young age, associated with germline heterozygous mutations of CDH1, MAP3K6 and CTNNA1 genes. In early stages it presents with non-specific and vague symptoms, in advanced stages it may cause nausea and vomiting, dysphagia, loss of appetite, abdominal mass or weight loss. Women have an increased risk of lobular breast cancer as well.

ORPHA:26106

Classification level: Disorder
  • Synonym(s):
    • FDGC
    • Familial diffuse cancer of stomach
    • Familial diffuse gastric cancer
    • HDGC
    • Hereditary diffuse cancer of stomach
    • Hereditary diffuse gastric adenocarcinoma
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: C16.9
  • OMIM: 137215
  • UMLS: C1708349
  • MeSH: -
  • GARD: 10900
  • MedDRA: -

  A summary on this disease is available in Deutsch (2018) Español (2018) Italiano (2018) Nederlands (2018) Français (2005)

Detailed information

General public

Guidelines

Disease review articles

Genetic Testing

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.